Dysembryoplastic neuroepithelial tumor: A rare brain tumor not to be misdiagnosed

Bhargava

Indian Journal of Neurosurgery

et al. 2020

Introduction Dysembryoplastic neuroepithelial tumor (DNET) is a rare cause of intractable epilepsy, which has excellent results in terms of seizure control after surgical resection. We present one such case, because of its rarity, to highlight the effect of tumor removal on seizure control, particularly DNET. Materials and Methods/Case Summary A 9-year-old male patient presented with sudden onset of partial seizures for the past 6 months. There were five episodes. In each episode, the patient engaged in irrelevant talk, followed by deviation of mouth to left and twitching movements. The episode lasted 5 minutes and there was no loss of consciousness. There was no aura or tongue bite, and in one of the episodes, the patient lost consciousness. There was no other significant positive history. On examination, the child was consciously alert, without focal neurological deficit or features of meningitis. There was no papilledema. The patient was on phenytoin sodium, phenobarbitone and clobazam. Magnetic resonance imaging (MRI) of brain was done with and without contrast. MRI revealed a lesion approximately 4.1 × 3.6 × 3.2 cm in the right medial temporal lobe. It was hypointense to brain on T1 and fluid-attenuated inversion recovery (FLAIR), and hyperintense on T2-weighted images. Diffusion restriction was present and there was minimal contrast uptake. There was no evidence of mass effect or midline shift After discussing the risks and benefits with parents, the patient underwent preanesthetic checkup, and was taken up for craniotomy and excision of tumor. Gross total excision was done. The child was started orally on day 1 postoperatively and ambulated. There were no further seizure episodes. The patient was continued on phenytoin and clobazam, and phenobarbitone was tapered gradually. At 6 months, the child was seizure-free. Conclusions DNET are rare tumors occurring early in life and presenting with intractable seizures. Surgical resection offers a good and safe chance for long-term seizure control.

Section: Discussionsupporting

confidence: 68%

“…FLAIR and apparent diffusion coefficient (ADC) may be helpful in distinguishing them. 3,4 Surgical resection of tumor is documented to be effective in long-term seizure control in cases of DNET. 1,2 It has been also found that it is safe in terms of mortality as well as morbidity.…”

Section: Discussionmentioning

confidence: 99%

Dysembryoplastic Neuroepithelial Tumor: A Rare Brain Tumor with Excellent Seizure Control after Surgical Resection

Bhargava

Indian Journal of Neurosurgery

et al. 2020

“…Similarly, dysembryoplastic neuroepithelial tumors are also well-circumscribed, indolent lesions amenable to complete resection with excellent long-term outcomes. Adjuvant therapy is utilized only for tumors which progress and are thought to be unresectable [84][85][86]. Gangliogliomas often harbor BRAF mutations, particularly BRAF V600E , or other alterations of the MAP kinase pathway [25,26].…”

Section: Benign Neuroepithelial Tumorsmentioning

confidence: 99%

Pediatric Low-Grade Gliomas

Collins

Pollack

2020

Cancers

Brain tumors constitute the largest source of oncologic mortality in children and low-grade gliomas are among most common pediatric central nervous system tumors. Pediatric low-grade gliomas differ from their counterparts in the adult population in their histopathology, genetics, and standard of care. Over the past decade, an increasingly detailed understanding of the molecular and genetic characteristics of pediatric brain tumors led to tailored therapy directed by integrated phenotypic and genotypic parameters and the availability of an increasing array of molecular-directed therapies. Advances in neuroimaging, conformal radiation therapy, and conventional chemotherapy further improved treatment outcomes. This article reviews the current classification of pediatric low-grade gliomas, their histopathologic and radiographic features, state-of-the-art surgical and adjuvant therapies, and emerging therapies currently under study in clinical trials.

“…They show neoplastic ganglion cells and perivascular lymphocytic infiltrate. 16 Best course of treatment is complete surgical resection which provides long term seizure control.…”

Section: Discussionmentioning

confidence: 99%

Dysembryoplastic neuropithelial tumor: a rare case report

Thanvi

Jangid²,

Joshi

2017

Int J Res Med Sci

Dysembryoplastic neuropithelial tumor (DNET) is a rare recently described, benign glioneural tumor frequently associated with intractable seizures in children and young adults which is important to recognise clinically and radiologically as it is surgically curable without need for adjuvant chemoradiotherapy. We hereby present a case report of a 10year old male child who presented with intractable seizures and right parietal space occupying lesion which was diagnosed DNET radiologically, treated by microsurgical excision and confirmed histopathologically as DNET, thus emphasising multidisciplinary role in management of this rare entity.